CHEMOTHERAPY FOR ADVANCED ESTHESIONEUROBLASTOMA - THE MAYO-CLINIC EXPERIENCE

OBJECTIVE: Esthesioneuroblastoma (olfactory neuroblastoma) is a rare n euroendocrine tumor that arises in the upper nasal cavity from the olf actory epithelium. Little information is available regarding the treat ment of these tumors with chemotherapy in the advanced setting. A retr ospective review of patients with recurrent esthesioneuroblastoma trea ted with chemotherapy between 1970 and 1995 at the Mayo Clinic was und ertaken to gain more information regarding the efficacy of chemotherap y treatment for these patients. METHODS: Ten patients were identified using a computerized data base available at this institution. The clin ical and pathological materials, when available, were reviewed, and ea ch tumor reviewed was assigned a Hyams' grade. RESULTS: There were six men and four women, ranging in age from 22 to 74 years, all of whom h ad assessable Kadish Stage C disease at the time of chemotherapy treat ment. The chemotherapy regimens and clinical follow-up varied during t his 25-year time span. The only tumor regression resultant from chemot herapy was observed in patients with high-grade tumors. Two of four pa tients with high-grade tumors obtained regression from first-line, pla tinum-based chemotherapy, with a mean duration of regression of 9.3 mo nths (range, 2-13 mo). Survival time from initial diagnosis was 139.3 months (range, 83-168 mo) in patients with low-grade tumors and 32.2 m onths (range, 5-84 mo) in patients with high-grade tumors. Survival fr om initial chemotherapy treatment was 44.5 months (range, 3-130 mo) in patients with low-grade tumors and 26.5 months (range, 2-67 mo) in pa tients with high-grade tumors. CONCLUSION: Hyams' grading of esthesion euroblastoma tumors seems to be important in predicting response to ch emotherapy. Despite sensitivity to platinum-based chemotherapy, patien ts with high-grade tumors in this series had a much more aggressive co urse than did those with lower-grade tumors. This series suggests that cisplatin-based chemotherapy is active in advanced, high-grade esthes ioneuroblastoma and is a reasonable choice in the systemic treatment o f these patients.